Shrestha, SundarBanepali, NirojSthapit, RakeshAgrawal, Dipesh2026-08-312026-08-312019https://hdl.handle.net/20.500.14572/7512Sundar Shrestha Department of General Surgery, Bir Hospital, NAMS, Kathmandu, Nepal Niroj Banepali Department of General Surgery, Bir hospital, NAMS, Kathmandu, Nepal Rakesh Sthapit Department of General Surgery, Bir Hospital, NAMS, Kathmandu, Nepal Dipesh Agrawal Department of General Surgery, Bir Hospital, NAMS, Kathmandu, NepalAbstract There are various cause of Primary amenorrhea in phenotypically females such as, complete androgen insensitivity syndrome, pure gonadal dysgenesis, 17b-hydroxysteroid dehydrogenase deficiency, or mixed gonadal dysgenesis. Primary amenorrhea in a phenotypically female is commonly encountered in Androgen insensitivity syndrome. In patients of AIS with intra-abdominal testis there is high chances of developing testicular tumour, among them Sertoli cell tumour and seminoma being the most common types. Leydig cell tumour in AIS is very rare and malignant leydig cell tumour is even further rarer. There are few case reported in the literatures of malignant leydig cell tumour with complete androgen insensitivity. Here we are reporting a case of 65 years married elderly patient with malignant leydig cell tumour with CAIS. Keywords: Complete androgen insensitivity syndrome; Leydig cell tumour; Testicular Feminization.en-USComplete androgen insensitivity syndromeLeydig cell tumourTesticular FeminizationArticle